Cervical Instability in Pierre Robin Sequence: An Addition to the Algorithm.

Area of Science:

• Craniofacial anomalies
• Pediatric surgery
• Orthopedic surgery

Background:

• Pierre Robin sequence (PRS) is a condition characterized by micrognathia, glossoptosis, and airway obstruction.
• Patients with PRS frequently present with associated congenital anomalies, particularly those affecting bone and cartilage development.
• Current management for PRS ranges from conservative measures to surgical interventions like distraction osteogenesis.

Observation:

• Surgical airway management in PRS patients, including nasal endoscopy or direct laryngoscopy, necessitates extreme neck extension.
• A case of syndromic PRS secondary to Stickler syndrome with a diagnosed cervical abnormality is presented.
• Advanced imaging, including 3D CT and dynamic lateral X-rays, was used to evaluate cervical instability.

Findings:

• The presented case highlights a syndromic PRS patient with an underlying cervical abnormality.
• Cervical spine instability can be present in PRS patients, particularly those with syndromic forms and connective tissue disorders.
• Standard intubation procedures may pose a significant risk to the cervical spine in these vulnerable patients.

Implications:

• Routine cervical spine evaluation should be incorporated into the preoperative assessment of all PRS patients.
• Early identification of cervical instability can guide surgical planning and prevent potential neurological complications.
• This approach is particularly important for PRS patients with suspected bone or collagen formation abnormalities, such as those with Stickler syndrome.